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Craniofacial and Tooth Biology > CTB 6 – Molecular regulation of developmental processes > Flashcards

CTB 6 – Molecular regulation of developmental processes Flashcards

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1
Q

What is the difference between the development of early or late defects with respect to craniofacial malformations?

A

Early developmental defects cause severe craniofacial malformations which are lethal, but later are less severe which allow survival

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2
Q

What is holoprosencephaly?

A

Insufficient division of the cerebral hemispheres

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3
Q

What is anencephaly?

A

Spina bifida

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4
Q

How is anencephaly prevented?

A

Folic acid

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5
Q

What is the main symptom in agnathia?

A

Mandible not present

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6
Q

What is the mildest form of cleft lip?

A

Cleft uvula

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7
Q

What is the most severe form of cleft lip?

A

Bilateral cleft lip and palate

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8
Q

What is ectodermal dysplasia?

A

Malformation of teeth

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9
Q

What is the main gene involved in the spectrum of facial midline disorders, ranging from holoprosencephaly and frontonasal dysplasia? How does the gene signal in these diseases?

A

SHH

Loss of SHH cell signalling in holoprosencephaly

Overactivation of SHH cell signalling in frontonasal dysplasia

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10
Q

What genetic factors can cause impaired early embryonic developmental processes?

A

Mutations in development genes, chromosomal abnormalities, abnormal gene regulation

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11
Q

What environmental factors can cause impaired early embryonic developmental processes?

A

Alcohol, smoking, drugs, etc….

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12
Q

What is gastrulation?

A

3 layers (ectoderm, mesoderm, endoderm) develop and fold to form a more complex organism

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13
Q

What derivative in the ectoderm forms craniofacial tissues?

A

Neural crest

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14
Q

What is organogenesis?

A

How body plan is established

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15
Q

What are the 4 types of cell communication?

A

Endocrine
Paracrine
Neuronal
Contact-dependent

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16
Q

What happens to a cell if it isn’t programmed to signal?

A

Cell dies

17
Q

What is the function of the phosphorylation cascade in cell signalling?

A

Induced by extracellular signal molecules to give out different cell responses

18
Q

What do signalling molecules induce?

A

Transcription factors

19
Q

What do transcription factors regulate?

A

Signalling molecules

20
Q

Name signalling molecules.

A

Fgf - fibroblast growth factors
Tgfß - transforming growth factor ß
Bmp - bone morphogenic proteins
Hh - hedgehog

21
Q

Name transcription factors.

A 
Hox - homeobox
Msx - muscle segment homeobox
Dlx - distal-less homeobox
Barx - barH-like homeobox
Pax - paired box
22
Q

What is the function of morphogenic gradients integrated at cellular level?

A

Regulate morphogenesis

23
Q

What are denticles?

A

Tooth-like projections

24
Q

Where is the opposing gradients of signalling molecules within the neural crest?

A

Neuroectoderm and epidermis

25
Q

What are the genes in the opposing gradients of signalling molecules within the neural crest?

A

Overlapping gradients of BMP4 and WNT6 signalling proteins

26
Q

What do the genes in the opposing gradients of signalling molecules within the neural crest induce expression of?

A

FoxD3 and slug

TF needed for cell specification and migration

27
Q

What protein does the Hox gene encode?

A

DNA-binding homeodomain

28
Q

What does Pax9 cause?

A

Oligodontia - more than 6 teeth missing

29
Q

What does Pax3 cause?

A

Waardenburg syndrome

30
Q

Where is Pax3 expressed?

A

Neural crest, muscle and CNS

31
Q

What are the symptoms of Waardenburg syndrome?

A
  • White hair colour
  • Wide-spaced eyes (hypertenlorism)
  • Short, broad nose
  • Epicanthal folds
32
Q

What are epicanthal folds?

A

Fold of the upper eyelid covering the inner corner of the eye

33
Q

What basic mechanism regulates organogenesis?

A

Reciprocal epithelial/mescenhymal interactions

34
Q

Within tooth development what happens in the initiation stage?

A
  • Overlapping gradients of signalling molecules, FGF and BMP in the dental epithelium
  • Induce transcriptional response, PAX9 and MSX1 in the dental mesenchyme
  • Determines tooth position
35
Q

Within tooth development what happens in the bud stage?

A
  • Dental mesenchyme secretes signalling molecules

- Induces formation of the enamel knot (non-dividing cells) in the dental epithelium

36
Q

Within tooth development what happens in the cap stage?

A
  • Enamel knot secretes signalling molecules that induces cell cycle arrest (BMP) within enamel knot cells, but induces cell proliferation (FGF) in surrounding cells
  • Determination of tooth shape

Craniofacial and Tooth Biology (6 decks)

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